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Systemic Capillary Leak Syndrome Successfully Treated with a TNF-A

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Systemic Capillary Leak Syndrome Successfully Treated with a TNF-A C al & ellu ic la n r li Im C m f u Journal of o n l o a Suyama et al., J Clin Cell Immunol 2014, 5:5 l n o r g u y o J DOI: 10.4172/2155-9899.1000266 ISSN: 2155-9899 Clinical & Cellular Immunology Letter to Editor Open Access Systemic Capillary Leak Syndrome Successfully Treated with a TNF-α Inhibitor Yasuhiro Suyama1*, Mitsumasa Kishimoto1, Ryo Rokutanda1, Chisum Min1, Gautam A Deshpande2, Youichiro Haji1, Ken-ichi Yamaguchi1, Akira Takeda1, Yukio Matsui1 and Masato Okada1 1Immuno-Rheumatology Center, St. Luke’s International Hospital, St. Luke’s International University, 9-1 Akashi-cho Chuo-Ku, Tokyo 104-8560, Japan 2Center for Clinical Epidemiology, St Luke’s Life Science Institute, 10-1 Akashi-cho Chuo-Ku, Tokyo 104-0044, Japan *Corresponding author: Yasuhiro Suyama, Immuno-Rheumatology Center, St. Luke's International Hospital, St. Luke’s International University, 9-1 Akashi-cho, Chuo-ku, Tokyo 104-8560, Japan, Tel: +81-3-3541-5151; Fax: +81-3-5550-6000; E-mail: [email protected] Received date: August 29, 2014, Accepted date: October 23, 2014, Published date: October 30, 2014 Copyright: © 2014 Suyama Y, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Letter blood pressure was observed. On the 22nd day of admission, he was discharged home without any sequellae. We read with interest the excellent article by Zhihui Xie et al., reporting in 35 patients with acute systemic capillary leak syndrome This is the first report of successful treatment of an adult case of (SCLS) the elevation of several cytokines, including TNFα, compared SCLS with the TNF-α inhibitor, infliximab. During acute attacks, SCLS to both baseline levels as well as sera from healthy controls [1].These is a potentially life-threatening disease due to both ischemic organ results suggest a possible breakthrough in treatment of SCLS, and failures from hypo perfusion during the extravasation phase, as well as support a previous report which showed similar increases in three flash pulmonary edema in the recovery phase [3]. The estimated five- patients with SCLS, as well as effectiveness of anti-TNF-α antibodies (6 year survival is reported to be 30-76% [4]. Limited data have suggested mg/kg) in the case of a 6-year-old boy [2]. Recently, our group also that a prophylactic regimen of terbutaline plus theophylline or experienced a similar case of SCLS occurring in an adult with TNFα intravenous immunoglobulin might decrease frequency of attacks [5]. elevations during acute attack, responding to TNF-α antagonist In addition, for treatment of the acute phase of life-threatening SCLS, (infliximab). some authors have reported using a combination of terbutaline and intravenous aminophylline [2] or high doses of IVIG [6]. However, as The patient, a 62-year-old Japanese man who had been prescribed fulminant acute attacks refractory to these therapeutic agents may an epinephrine auto-injector for idiopathic anaphylaxis characterized occur, establishing optimal salvage therapy is necessary. TNF-α, by recurrent episodes of nausea, peripheral edema, and hypovolemia, known to stimulate the release of endothelial cytokines and increase was admitted to our hospital with hypotension. He reported no vascular permeability, may play a role [7]. As such, our patient has also allergies to foods or medications and reported no unusual exposures. continued to receive infliximab as maintenance therapy every 2 On examination, his systolic blood pressure was 75 mmHg, pulse rate months. Although he experienced a recurrent acute attack was 58 beats/min, and temperature was 36.4. Neither wheezing nor immediately prior to a subsequent infusion, as well as an isolated rashes were observed. He was admitted to the intensive care unit due attack occurring 4 days after an infusion, serum TNF level were noted to insufficient response to initial treatment with 0.3 mg of to be elevated in both of these instances at 12.5 pg/ml and 11.7 pg/mL, intramuscular epinephrine, 3.5 Lof intravenous fluids, and 500 mg of respectively (normal range, <1.79 pg/mL); administration of additional intravenous hydrocortisone. Based on his severe shock status, infliximab (3 mg/kg) lead to quick recovery in both episodes. Zhihui hemoconcentration (hematocrit, 57.3%), hypoalbuminemia (serum Xie et al., also report a 45 year-old male on infliximab for maintenance albumin, 1.7 g/dL), lack of identifiable triggers despite intensive therapy [1]. Our case, in combination with the previous paediatric investigation, and presence of a monoclonal gammopathy (IgG kappa) report, strongly suggests that TNF inhibitors may be an excellent already noted at a previous hospital, the patient was diagnosed with therapeutic option for both the induction and maintenance treatment SCLS. The acute attack was managed with extensive fluid resuscitation of refractory SCLS. and continuous administration of adrenaline; he was subsequently discharged in good condition with a prophylactic regimen of terbutaline plus theophylline. Twelve days after discharge, he was Acknowledgement readmitted for a recurrent episode of acute, anaphylaxis-like We thank Dr. Masatomo Kiyota (Department of General Internal hypotension. Despite treatment with 12 L of intravenous fluids, in Medicine, AsoIizuka Hospital, Fukuoka, Japan), who provided expert addition to adrenaline, dopamine at 0.35 μg/kg/minute, two 250 mg clinical advice, and Dr. Masahiro Uni (Department of Hematology & doses of hydrocortisone, an H2 blocker, leukotriene modifying Oncology, The University of Tokyo Hospital), who performed a therapy, and theophylline, his condition did not improve. On the perfect cardiopulmonary resuscitation. second day of admission, he was intubated due to respiratory failure. Worsening hemoconcentration (hematocrit, 59.3%), References hypoalbuminemia (serum albumin, 1.5 g/dL), and renal failure did not respond to the additional infusion of IVIG (400 mg/kg). The patient 1. Xie Z, Chan E, Yin Y, Ghosh CC, Wisch L, et al. (2014) Inflammatory subsequently went into pulseless electrical activity, requiring six cycles Markers of the Systemic Capillary Leak Syndrome (Clarkson Disease). J of cardiopulmonary resuscitation before pulse was recovered. Given Clin Cell Immunol 5: 213. his persistent shock status (pH 6.8; lactate 14 mmol/L), he was given 2. Dowden AM, Rullo OJ, Aziz N, Fasano MB, Chatila T, et al. (2009) Idiopathic systemic capillary leak syndrome: novel therapy for acute infliximab at 3 mg/kg. Although his condition did not respond to the attacks. J Allergy Clin Immunol 124: 1111-1113. initial infusion of infliximab, a second dose of 3 mg/kg, given 30 3. Druey KM, Greipp PR (2010) Narrative review: the systemic capillary minutes later, elicited a dramatic clinical response and increase of leak syndrome. Ann Intern Med 153: 90-98. J Clin Cell Immunol Volume 5 • Issue 5 • 1000266 ISSN:2155-9899 JCCI, an open access journal Citation: Suyama Y, Kishimoto M, Rokutanda R, Min C, Deshpande GA, et al. (2014) Systemic Capillary Leak Syndrome Successfully Treated with a TNF-α Inhibitor. J Clin Cell Immunol 5: 266. doi:10.4172/2155-9899.1000266 Page 2 of 2 4. Dhir V, Arya V, Malav IC, Suryanarayanan BS, Gupta R, et al. (2007) 6. Lambert M, Launay D, Hachulla E, Morell-Dubois S, Soland V, et al. Idiopathic systemiccapillary leak syndrome (SCLS): case report and (2008) High-dose intravenous immunoglobulins dramatically reverse systematic review of cases reported in thelast 16 years. Intern Med 46: systemic capillary leaksyndrome. Crit Care Med 36: 2184-2187. 899-904. 7. Vadlamani L, Iyengar S (2004) Tumor necrosis factor alpha 5. Gousseff M, Arnaud L, Lambert M, Hot A, Hamidou M, Duhaut P, et al. polymorphism in heart failure/cardiomyopathy. Congest Heart Fail 10: (2011) The systemic capillary leak syndrome: a case series of 28 patients 289-292. from a European registry. Ann Intern Med 154: 464-471. J Clin Cell Immunol Volume 5 • Issue 5 • 1000266 ISSN:2155-9899 JCCI, an open access journal.
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